Background Adult onset Still's disease (AOSD) is a rare systemic inflammatory condition of unknown etiology, which in long-term can occasionally be complicated by AA-amyloidosis.
Objectives To present a case of clinical and histopatholohical regression of AA-amyloidosis in patient with AOSD.
Methods Prospective 5-year follow up of a patient, b.1969, with AOSD complicated by AA-amyloidosis.
Results Based on case medical records the patient presented in 1999 y. with high fever up to 40°C, profuse sweat, eruption of salmon-pink papular rash on peak of fever, disappearing after spike resolution, swallowing pain, enlargement of subclavian and axillary lymph-nodes, and painful wrists. A diagnosis of AOSD was established. Prednisone at 40 mg/day was started in 2000 y. with gradual GC dose tapering up to 10 mg/day, on which patient stayed for a long time. Febrile fever persisted until 2003, and was alleviated by additional intake of nimesulide 100 mg/day. Gradually fever episodes became rare, but starting from February 2006 the patient noticed swelling of lower limbs, and proteinuria up to 3 g/l was documented. The patient was hospitalized in October 2006y. to the Institute of rheumatology with high lab activity of the disease (CRP 60 mg/l), nephrotic syndrome (peripheral edema, total protein 50,4 g/l, albumin 16,6 g/l, cholesterol 13,7 mmol/l, proteinuria 2,27 g/day). Amyloid deposits were found in biopsy samples of duodenal mucous (Fig. 1a) and small salivary glands (Fig. 1b). Chlorambucil was initiated at 6 mg/day, further - 8 mg/day, OGC dose was increased to 20 mg/day prednisone. Chlorambucil was discontinued in June 2007 due to frequent recurrence of Herpes zoster infection. In September 2007 leflunomide was administered at 20 mg/day as a DMARD. One month later the patient stated pain improvement in her wrists and became afebrile. By December the patient stated reduction in leg edema, and from March 2008 - complete resolution of edema. The OGC dose was gradually tapered starting from January 2008 up to the final dose of 2,5 mg/day, leflunomide – to 10 mg/day. The patient became asymptomatic, nephrotic syndrome and proteinuria resolved, normal blood levels of total protein, albumin and cholesterol, normalized ESR and leukocyte count. Amyloid deposits were still present in duodenal mucous and salivary glands biopsy samples. The patient continued with prescribed therapy, her condition stabilized. After 5 years of therapy the patient underwent through check up in an in-hospital setting (2013), her lab tests revealed normal levels of total protein, albumin, RP, ESR, SAA <9,4 mg/l. There were no amyloid deposits in duodenal and salivary gland samples (Fig. 2a, 2b).The patient continues 2 mg/day methypred 10 mg/day leflunomide.
Conclusions Presented case demonstrates crucial importance of underlying rheumatic disease remission in achieving clinical and histopathological regression of AA-amyloidosis, even at nephrotic stage.
Disclosure of Interest None declared